Protoporphyrin-induced photohemolysis in protoporphyria and in normal red blood cells

Schothorst, A.A.; Steveninck, J. Van; Went, L. N.; Suurmond, D.

doi:10.1016/0009-8981(70)90158-0

Cited by 110 publications

(29 citation statements)

References 10 publications

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“…These authors hypothesized that red cell membrane damage caused by the light-induced excitation of protoporphyrin was due to the production of free radicals or hydrogen peroxide, which in turn resulted in the oxidation of unsaturated fatty acids (UFA) in the cell membrane. Studies in this laboratory, and by others, demonstrating that EPP photohemolysis will not occur in the absence of oxygen are consistent with Ludwig's hypothesis (7,8).…”

supporting

confidence: 89%

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Erythropoietic Protoporphyria: Lipid Peroxidation and Red Cell Membrane Damage Associated with Photohemolysis

Goldstein¹,

Harber²

1972

J. Clin. Invest.

169

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A B S T R A C T The mechanism by which long wavelength ultraviolet light hemolyzes red cells obtained from patients with erythropoietic protoporphyria (EPP) was investigated. Previous studies had suggested that irradiation of these red cells with wavelengths of light capable of eliciting dermatological manifestations led to oxygen-dependent colloid osmotic hemolysis through the formation of peroxides. In the present report, lipid peroxidation during in vitro irradiation of EPP red cells with long ultraviolet light was demonstrated by: (a) the formation of 2-thiobarbituric acid reactants; (b) the presence of conjugated diene bonds in red cell lipid; and (c) the selective loss of unsaturated fatty acids proportional to the number of carbon-carbon double bonds in each. Irradiation of EPP red cells was also shown to result in the formation of hydrogen peroxide.Before photohemolysis there was a decline in cell membrane sulfhydryl groups and a loss in activity of the cell membrane enzyme acetylcholinesterase. These parameters provide further evidence suggesting that the cell membrane is a primary site of the photohemolytic effect of long ultraviolet light in EPP red cells.Further evaluation of the radiation-induced inactivation of EPP red cell acetylcholinesterase was performed by radiating mixtures containing bovine erythrocyte acetylcholinesterase and protoporphyrin IX. These studies revealed that the rate of decline in enzyme activity is accelerated by the addition of linoleic acid, an unsaturated fatty acid, but not by palmitic acid, a saturated fatty acid.

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confidence: 89%

“…The pathway by which the interaction of light and protoporphyrin might result in cell damage has been studied in this and other laboratories by investigation of EPP red cells subjected to radiation in vitro (3)(4)(5)(6)(7)(8).…”

Section: Introduction Erythropoietic Protoporphyria (Epp)' Is An Inhementioning

confidence: 99%

Erythropoietic Protoporphyria: Lipid Peroxidation and Red Cell Membrane Damage Associated with Photohemolysis

Goldstein¹,

Harber²

1972

J. Clin. Invest.

169

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“…These simple membrane model systems have continued to be investigated by numerous investigators since that time (e.g. Goldstein and Harber, 1972;Schothorst et al, 1970;Girotti, 1976;Valenzeno and Pooler, 1982a). Today such studies have been supplemented by investigations using synthetic model membrane systems such as micelles, liposomes and vesicles.…”

Section: Introductionmentioning

confidence: 99%

Photomodification of Biological Membranes With Emphasis on Singlet Oxygen Mechanisms

Valenzeno

1987

Photochem & Photobiology

263

118

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Abstract-This review discusses photomodification of biological membranes and model membrane systems. Current concepts of membrane structure are first reviewed briefly. The role of preillumination association of sensitizer with membranes as it relates to photomodification rate is discussed, as well as the role of singlet oxygen in membrane photomodification. Finally the characteristics of singlet oxygen generation in membranes are considered. The evidence clearly indicates that membrane photomodification cannot be understood based only on the properties of sensitizers and singlet oxygen in aqueous solution. Rather the properties of sensitizers in association with membranes are the determinants of membrane photomodification. These properties differ significantly in aqueous solution and in membranes.

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“…Irradiation of erythrocytes from patients with erythropoietic protoporphyria results in photohemolysis of the cells (1,2). This photohemolytic process has been characterized in a number of ways (3,4).…”

Section: Introductionmentioning

confidence: 99%

Light-induced protoporphyrin release from erythrocytes in erythropoietic protoporphyria.

Sandberg¹,

Brun²

1982

J. Clin. Invest.

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A B S T R A C T The photohemolysis of normal erythrocytes incubated with protoporphyrin is reduced in the presence of albumin. When globin is added to normal erythrocytes loaded with protoporphyrin, protoporphyrin is bound to globin. During irradiation protoporphyrin moves from globin to the erythrocyte membrane and photohemolysis is initiated.Erythrocytes in patients with erythropoietic protoporphyria contain large amounts of protoporphyrin bound to hemoglobin. Upon irradiation of these cells in the absence of albumin, 40% of protoporphyrin and 80% of hemoglobin is released after 240 kJ/m2. The released protoporphyrin is hemoglobin bound. In contrast, when albumin is present only 8% of hemoglobin is released whereas protoporphyrin is released to 76%. The released protoporphyrin is albumin bound.A hypothesis for the release of erythrocyte protoporphyrin in erythropoietic protoporphyria without simultaneous hemolysis is proposed: Upon irradiation protoporphyrin photodamages its binding sites on hemoglobin, moves through the plasma membrane, and is bound to albumin in plasma.

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Protoporphyrin-induced photohemolysis in protoporphyria and in normal red blood cells

Cited by 110 publications

References 10 publications

Erythropoietic Protoporphyria: Lipid Peroxidation and Red Cell Membrane Damage Associated with Photohemolysis

Erythropoietic Protoporphyria: Lipid Peroxidation and Red Cell Membrane Damage Associated with Photohemolysis

Photomodification of Biological Membranes With Emphasis on Singlet Oxygen Mechanisms

Light-induced protoporphyrin release from erythrocytes in erythropoietic protoporphyria.

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